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Common Pediatric Solid Tumors

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Pediatric Surgery Elective Course

College of Medicine, King Faisal University

Al-Ahsa, Saudi Arabia

Published in: Health & Medicine
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Common Pediatric Solid Tumors

  1. 1. Common Pediatric Solid Tumors Abdullatiff Sami Al-Rashed College of Medicine, King Faisal University Al-Ahsa, Saudi Arabia
  2. 2. Objectives • Definition. • Epidemiology. • Etiology. • Types. • Symptoms & Signs. • Staging. • Diagnosis. • Treatment. • Prognosis. • Definition. • Epidemiology. • Etiology. • Types. • Symptoms & Signs. • Staging. • Diagnosis. • Treatment. • Prognosis. • Definition. • Treatment.
  3. 3. Lymphoma (Hodgkin's & Non-Hodgkin's) Lymphoma (Hodgkin's & Non-Hodgkin's)
  4. 4. Definition • Neoplasia of the lymphatic system and its precursor cells with genetically distrusted regulation of proliferation, differentiation and apoptosis. • Characterized by painless, progressive enlargement of the lymph nodes with continues extension between lymph node regions.
  5. 5. Epidemiology • Lymphoma accounts 10% of all pediatric neoplasia. Hodgkin's lymphoma Non-Hodgkin's lymphoma 7 in 1 million children < age of 16 newly diagnosed with HL annually Ratio of boys to girls 2:1 Boys > Girls , but during adolescence the incidence is the same Peak incidence between 5 to 15 years old Peak incidence between 15 to 35 years old
  6. 6. Etiology • Unknown etiology in humans. • It has been found that there is correlation with infection “e.g Epstein-barr Virus”. • Correlation with Socioeconomic status: the higher SES the more frequently HL occur. • Genetic causes. • Unknown etiology in humans. • Predisposing factors include: – Autoimmune disorders. – Infections ( HIV, EBV, HTLV-1 ) – Congenital B-Cell defect. – Congenital T-Cell defect. – Drug induced (immunosuppressive treatment) HL NHL
  7. 7. Types Lymphoma Hodgkin's lymphoma Classical Hodgkin’s lymphoma (90- 95%). Nodular lymphocyte predominant HL Non Hodgkin's lymphoma Diffuse large B- cell lymphoma (30%) Peripheral T-call lymphoma Burkitt Lymphoma (Highly aggressive) Other types
  8. 8. Symptoms & Signs Painless enlarged lymph nodes (commonest cervical)
  9. 9. Symptoms & Signs
  10. 10. Staging
  11. 11. Diagnosis
  12. 12. Treatment • Treatment plan is assigned based on the type and stage of lymphoma. • Traditionally, management consists of a combination of chemotherapy drugs. • Combination of radiotherapy and chemotherapy can be used in Hodgkin's lymphoma.
  13. 13. Prognosis
  14. 14. Nephroblastoma (Wilms tumor) Nephroblastoma (Wilms tumor)
  15. 15. Definition • Malignant embryonal tumor of renal tissue. • First described in 1899 by max wilms.
  16. 16. Epidemiology
  17. 17. Etiology • Wilms tumor has been associated with loss of function mutations of a number of tumor suppressor and transcription genes. • These include mutations of the WT1, p53, FWT1, and FWT2 genes, and at the 11p15.5 locus. • Associated with some congenital syndromes. These syndromes include: – WAGR syndrome. – Denys-Drash syndrome. – Beckwith-Wiedemann syndrome.
  18. 18. Types • Histologically, the classic favorable histology Wilms tumor is comprised of three cell types:
  19. 19. Symptoms & Signs
  20. 20. Staging
  21. 21. Diagnosis
  22. 22. Diagnosis THE DEFINITIVE DIAGNOSIS OF WILMS TUMOR IS MADE BY HISTOLOGIC CONFIRMATION AT THE TIME OF EITHER SURGICAL EXCISION OR BIOPSY.
  23. 23. Treatment • Treatment plan is assigned based on results of the initial staging, histological, and molecular studies. • In General , lines of treatment include: Surgery Chemotherapy Radiotherapy
  24. 24. Prognosis
  25. 25. NeuroblastomaNeuroblastoma
  26. 26. Definition • Malignant embyronal tumor of precursor cells of sympathetic ganglia and adrenal medulla. • Commonly used to refer to a spectrum of neuroblastic tumors (including neuroblastomas, ganglioneuroblastomas, and ganglioneuromas)
  27. 27. Epidemiology
  28. 28. Etiology • Etiology is unknown.  Risk factors: • Maternal factors: These include the following: – Opiate consumption – Folate deficiency – Toxic exposures – Congenital abnormalities – Gestational diabetes mellitus • Genetic factors: – A higher incidence of neuroblastoma has been suggested in girls with Turner syndrome, Hirschsprung's disease, central hypoventilation, and neurofibromatosis type 1 (NF1)
  29. 29. Types • Neuroblastomas can arise anywhere throughout the sympathetic nervous system. • The common primary sites: Adrenal gland (40%) Abdominal (25%) Thoracic (15%)
  30. 30. Types
  31. 31. Symptoms & Signs
  32. 32. Staging
  33. 33. Diagnosis
  34. 34. Diagnosis • Diagnostic criteria — Minimum criteria for establishing a diagnosis of neuroblastoma have been agreed upon by an international consensus panel. A definitive diagnosis of neuroblastoma requires one of the following: – An unequivocal histologic diagnosis from tumor tissue by light microscopy, with or without immunohistochemistry, electron microscopy, or increased urine (or serum) catecholamines or their metabolites. – Evidence of metastases to bone marrow on an aspirate or trephine biopsy with concomitant elevation of urinary or serum catecholamines or their metabolites.
  35. 35. Treatment • Treatment plan is assigned based on: – Stage of the disease. – Patient age. – Histologic appearance of the tumor. – Presence or absence of amplification of the MYCN oncogene. – Quantitative DNA content of the tumor (DNA index or ploidy).
  36. 36. Treatment •In General , lines of treatment include: Surgery Chemotherapy Radiotherapy
  37. 37. Prognosis
  38. 38. hepatoblastomahepatoblastoma
  39. 39. Definition • Hepatoblastoma is the most common primary hepatic malignancy in early childhood. • The majority of hepatoblastomas occur in the first two years of life and rarely in children older than five years.
  40. 40. Epidemiology • One percent of all pediatric neoplasias • The incidence of hepatoblastoma in boys is twice that in girls.
  41. 41. Etiology • Exact etiology is unknown. • Syndromes with an increased incidence of hepatoblastoma include: – Beckwith Wiedmann syndrome. – Trisomy 18 & 21. – Acardia syndrome. – Li-Fraumeni syndrome. – Goldenhar syndrome (a type of craniofacial microsomia). – Type 1a glycogen storage disease (von Gierke’s disease). – Familial adenomatous polyposis (FAP).
  42. 42. Types
  43. 43. Symptoms & Signs
  44. 44. Staging Stage Characteristics Stage I • The tumor is completely resectable via wedge resection or lobectomy. • The tumor has PFH results. • The AFP level is within reference range within 4 weeks of surgery. Stage IIA • The tumor is completely resectable. • The tumor has histologic results other than PFH (UH). Stage IIB • The tumor is completely resectable. • AFP findings are negative at time of diagnosis (ie, no marker to follow). Stage III (any of the following) • The tumor is initially unresectable but is confined to one lobe of liver. • Gross residual disease is present after surgery. • Tumor ruptures or spills preoperatively or intraoperatively. • Regional lymph nodes are involved. Stage IV Distant bone or lung metastasis is present.
  45. 45. Diagnosis
  46. 46. Treatment • Treatment plan is assigned based on results of the initial staging, histological, and molecular studies. • In General , lines of treatment include: Surgery Chemotherapy Radiotherapy
  47. 47. Prognosis
  48. 48. HemangiomaHemangioma
  49. 49. Definition • Infantile hemangiomas are benign vascular neoplasms that have a characteristic clinical course marked by early proliferation and followed by spontaneous involution. • Hemangiomas are the most common tumors of infancy and usually are medically insignificant.
  50. 50. Treatment • The vast majority of infantile hemangiomas do not require any medical or surgical intervention. • Intervention may be required for lesions with potential to interfere with a vital structure or function. These include, but are not limited to: – Lesions in the airway, liver, or gastrointestinal tract. – Lesions in the periorbital region. – Very large, rapidly growing cutaneous hemangiomas.
  51. 51. Treatment • Treatment choices are: – Drugs: Steroids and Beta-Blocker – Surgery: laser surgery or surgical excesion
  52. 52. References

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